Naxos disease is an autosomal recessive disorder that combines palmoplantar keratoderma and other ectodermal features with cardiac disorders suggesting arrhythmogenic right ventricular dysplasia/cardiomyopathy. It was first reported in families on the Greek island of Naxos by Protonotarios (1986). At that time the authors described 4 families and 9 cases. Seven of the 9 affected persons showed signs and symptoms of heart disease, including cardiomegaly, EKG abnormalities, episodes of ventricular tachycardia, and, in 1 patient, sudden death. All patients had echocardiographic evidence of enlargement of the right ventricle and a right ventricular band; in 3, the left ventricle was also affected. Protonotarios et al. (1986) pictured the scalp hair, which was described as dense, rough, and bristly, resembling steel wire.

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